NBME 20 Ovaries (NBME Answers)

NBME 20 Answers

nbme20/Block 2/Question#43 (reveal difficulty score)
An 18-year-old woman comes to the physician ...
Ovaries 🔍 / 📺 / 🌳 / 📖
tags: repro

+6 upvote downvote
submitted by ∗step420(32)

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This is mullerian agenesis. Normal ovaries but absent uterus.

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endochondral why not androgen insensitivity? +

shaeking I was wondering the same thing because doesn't androgen insensitivity also have normal female secondary characteristics. Was it the levels of hormones because she doesn't have abnormally high testosterone? +3

swb Androgen insensitivity has the same presentation and symptoms. What's the clue that it is mullerian agenesis instead ? +27

sugaplum Testosterone would be high if it was androgen insensitivity FA 2019 Pg 625 +22

charcot_bouchard Testo would be high in AIS. in AIS pubic hair, axillary hair doesnt devlop because of androgen insensitivity. both have normal breast dev and primary amenorrhea +2

dickass This is not androgen insensitivity because she has perfectly normal Estradiol, which means she has perfectly normal ovaries. She also has regular female levels of testosterone. +5

rockodude thank you @dickass +1

j44n Also AIS has paradoxically large boobs-> tanner stage 5 and thats not mentioned anywhere +

fatboyslim Androgen insensitivity syndrome will have high testosterone because the testosterone receptor isn't functional, therefore the body is like what the heck and starts dumping more testosterone trying to make them a male but it doesn't work unfortunately +

+5 upvote downvote
submitted by ∗sympathetikey(1600)

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Mullerian Agenesis (aka: Mayer-Rokitansky-Kuster-Hauser Syndrome)

Underdevelopment of the Mullerian system leading to congential absence of the vagina. Usually no cervix or uterus.

May present as 1° amenorrhea (due to a lack of uterine development) in females with fully developed 2° sexual characteristics. Functional ovaries allow for normal sexual characteristics and hormone levels.

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+2 upvote downvote
submitted by ∗temmy(152)

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i think it is not androgen insensitivity because the testosterone levels are normal. They should be high in AIS.

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+1 upvote downvote
submitted by ∗imgdoc(183)

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I know it's tempting to think that this may be Androgen insensitivity syndrome but its not, for it to be AIS there needed to be an increase in testosterone. In this case it was in the normal range for a female. Now that we know the person is definitely female -> Mullerian agenesis becomes the best answer.

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+1 upvote downvote
submitted by ∗topgunber(67)

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Rokitansky Kuser Hauser syndrome.

Hormones are normal as well as gonads.

INTERNAL GENITALIA are malformed (mainly uterus/tubes). Uterus can also be unicornate or there can be didelphysis or whatever that word is. though you don't need to know that long name by heart it helps with speed. Understand: Hormone profile is normal. If this patient had testes, the testosterone and estrogen would likely be opposite.

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topgunber right theres a mayer somewhere in that disease name... ffs +1

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